I'm Aware That I'm Rare: Liz DeVivo
In 2000, Liz DeVivo was diagnosed with Scleroderma, a rare and chronic autoimmune disease. The disease attacked her internal organs, causing pulmonary hypertension. Due to the urgency of her condition, she was placed at the top of the transplant list in September 2001. Liz wrote a book (Holding On For Dear Life), chronicling her journey as a young mother to a heart and double lung transplant. Her transplant story was also featured in a commercial in the Super Bowl 51 broadcast.
My name is Liz DeVivo, and I live in Upstate New York in Binghamton, New York.
I first started having changes to my body in 1996, when I was pregnant with my second child. The changes came kind of slowly, and [my doctors] noticed that I had an elevated antinuclear antibody level, an ANA level, and they thought that that would go away after I had my baby.
After I had my baby, it did not go away, and then other changes started to happen. I started to have Raynaud’s phenomenon, and I started to have changes to my hands and face where my skin started to harden and to thin and to kind of become shinier. My hands were swelling and my joints started to get arthritic. Those were the first changes I noticed.
It probably took about two years before they were able to diagnosis it as scleroderma, which is an autoimmune disease much like lupus or MS or those types of more familiar autoimmune diseases. Scleroderma actually means hardening of skin. What happens is your body overproduces collagen. It can happen to your skin outside of your body, and it can happen inside your body to your organs.
When I was first diagnosed, it was really looking like it was mostly skin involved outside, and so I didn’t really pay much attention to it. I felt okay. I had some fatigue, but I was okay. I had two little kids. I was going to college. I was tired. I just assumed that it was because I was a full-time grad student and taking care of two kids that I was exhausted and kind of out of shape.
I found myself getting more and more out of breath, and I really chocked it up to inertia and lack of doing things. I did two weeks of exercise and realized I wasn’t getting any exercise tolerance. I was actually getting worse. I knew I was in a bit of trouble then… that something was going on.
I ended up going to the doctor, and my blood pressure was really high. They decided to send me to the hospital, and when they did send me to the hospital I had a pericardial effusion, which is fluid around your heart that builds up in the sac of your heart. That was the first thing that happened that kind of threw me into crisis mode, and I knew I wasn’t well and something was wrong.
After that, they really thought that was a scleroderma-based event, and they thought I would heal and go on, but I just didn’t heal and I couldn’t get my breath back. I was really struggling with walking, even just a few yards or a few feet. It was really hard. Actually, I kept thinking that I would get better and I would go back to work and I would be fine. It just wouldn’t turn around.
Finally they sent me to a cardiologist, and when I went to the cardiologist he said, “I’m sorry. You have pulmonary hypertension. Do you know what that is?” I did know because I knew that that was a complication for some people with scleroderma. He just looked at me and he said, “I’m sorry.” I said, “Well, what’s my prognosis?” He said, “Maybe two to five years,” and that was literally the end of the discussion. That was all the discussion there was at the time, and that was the year 2000.
I was devastated after talking to him. I was angry after talking to him. I was sad after talking to him. But mostly I was just determined to figure out something. I just couldn’t believe that that was just it.
The first thing I did was go to New York City and find a pulmonary hypertension specialist in New York. At that time there was very limited help. There was very limited help on the computer. There weren’t very many support groups. There was very limited stuff going on. You could find a tiny bit, but not much. I went to New York hoping someone could give me some kind of hope. I met with a pulmonary hypertension specialist there, and he did all the preliminary stuff, the heart catheterization, the echos, and everything that goes along with that.
Basically, my pulmonary pressures were really high and I was a pretty good wreck. I think it was in the 120s, when I was first diagnosed and I could barely walk. I was blue. My lips were blue, my fingers were blue, my feet were blue. I was pretty blue.
That was in July of 2000, and by September of 2000 I was put on IV medication, and they thought that perhaps they could add something to stop the fibrosis or the hardening of my lungs, so I also did six months of chemotherapy to help stop the hardening of my lungs. All through that time, I still had the pericardial effusions, so my heart kept filling up and I kept having to have … I’d like get to the point of almost cardiac tamponade. Then they would drain it.
After the third time it happened, they finally decided to just open up my pericardial sac and leave it open, which was helpful. That was definitely helpful. That first year that I was diagnosed was really hard. I was hospitalized over and over and over. It was like plugging a dike. Every time you’d plug the dike, it would spring a leak somewhere else, something else would go wrong. I knew I was running out of time fast. The writing was on the wall pretty clear. I knew I needed to start looking for other solutions.
I went to a hospital in New York and met with the surgeon there, and he promptly turned me down right there on the spot, right to my face, and told me I was too sick and I was a waste of resources, is how he put it. He didn’t think I could survive the illness, and because it was scleroderma, and scleroderma is not even curable with a transplant, he couldn’t justify the use, I guess. So I was pretty devastated, completely devastated, but very determined still.
I had a lot of support from my family and friends, and we just set about calling and finding out what centers in the U.S. would even consider me. At that time there were only like three places that would consider me in Texas and in California, and in Pittsburgh. Because Pittsburgh is the closest, I started with them. I called them and made the appointment thinking maybe I needed a lung or two, and they said, “No, you need a heart and two lungs.” I knew that that was just going to be a harder surgery physically and a harder surgery to wait for. Those are very rare surgeries with very few people on the list. I knew my chances were diminishing fast, and at the time I was only about 95 pounds.
I knew I was waiting for a very small donor, because you can put small organs in a big body, but you can’t put big organs in a small body. I was waiting for a small person, and I was a B- blood type, which is pretty rare, so I knew that my odds were not really very good at all. I waited 15 months before I got my first call for a transplant. I was in New York City for an educational forum on scleroderma, and it was November, and who would figure, but it was actually snowy and icy and windy, and I couldn’t get a plane to fly me to Pittsburgh. I had to turn down the organs, which was horrible, but I had to believe that they were for someone else. They just had to be for someone else.
Then I continued my wait, and at 23 months, I finally got the call, the real deal call. It was in July, July 29th of 2003, and it was a beautiful sunny day. I went to Pittsburgh and I was not even afraid at that point because I knew it was my only chance to live. My surgery itself went pretty well. It was pretty standard, I think, about maybe eight hours. My surgeon came out and told my family that everything went well, and now they were just going to work on closing me up and he would be out in a few hours to let them know. I guess the few hours turned into many hours and turned into more hours. I had some problems with bleeding, and they couldn’t stop my heart from bleeding, and they couldn’t get my heart in sync either. I have what’s called a clamshell incision, which goes from my armpit to my armpit, and they literally work on me like that, like open. This part is really hard for me to really even fathom or understand.
They worked on me for a week while I was open like that, which was just crazy to even imagine. I was in a medically-induced coma for at least a few weeks, if not more, before I was conscious. Even when I was conscious I had no idea what was going on. I was hallucinating like crazy. I was in the ICU for like seven weeks. I just had a really hard time.
Finally, when I got out of surgery and finally when I got out of the ICU, it probably took another couple months or so before I actually made it home. I had my transplant in July and I got home the day before Thanksgiving. It was a pretty long road. It was not easy. Even at that point, I still didn’t even know I was better. Everyone would say, “Liz, you’re pink. Your lips are pink. You look good,” and I couldn’t even tell that I was better. It took like six months after my surgery, and we were walking and I remember thinking, “My God, I think that wasn’t so hard.” It was like the light bulb went off in my head and I couldn’t even believe it. “I think I’m better.”
From that moment on the joy just started rushing in and I just started to heal faster and faster, and feel better and be better. It was a pretty amazing journey for sure. I think I took about a whole year to really recoup from that surgery, for sure. My kids were pretty little at that point. They were 7 and 10, so I was pretty happy to be their mother and to take care of the, and to take care of my family, and honored to do those things. My kids graduated from high school. I returned to work part-time as a social worker, and I worked in dialysis for about 4-5 years doing that, which was wonderful.
I spend my time volunteering for Donate Life events, and educating the public about organ donation, and I also spend time talking to medical health professionals. I spend a lot of time talking to nurses, new hires, and fellows and interns when they’ll have me.
I think the most important thing health professionals can give us is hope. That’s like the most important asset, as far as I’m concerned, that anybody can give somebody who is suffering from a chronic illness is hope. That’s why it’s important to share those stories.
The biggest asset anyone has in their fight to any disease is their brain, and that’s the bottom line. If you believe it can happen, if it’s possible, if you believe it’s possible, it can be possible. If you don’t think it’s possible because all the data says it’s not possible, it will never be possible.
I started writing a blog while I was sick, and it ended up becoming a book. My book is called Holding On For Dear Life: What My Fatal Illness Taught Me About Living. It started because people kept saying, “Liz, you need to make this a book. I’ve learned so much from you. You really need to make this a book.” It was more than just people who are sick even. It was all kinds of people that were telling me that it had some importance to them in their life.
I added more to the blog, so it’s more than just a blog, and it became health advice for people going through catastrophic illness, and it also became like a primer of some kind for healthcare professionals, too, so it has some advice for healthcare professionals. It’s almost like a How To Survive Your Catastrophic Illness book. It talks about we’re more than just our illness.
We come to the table as patients, and we come with all our symptoms, but we also come with all our history and our family members and everything else that comes to the table. We’re more than just the symptoms that you add up in the column. It really is about hope and the importance of hope in the healing process.
The medicines are changing every minute, every day, every year. It’s amazing the strides that have taken place in the last 10 years, 15 years. It’s mind-boggling. When I see the stories of people who are newly diagnosed, I’m grateful for them because I know it’s so much better than it was. My takeaway is to never forget that your hope and your brain, it’s the greatest asset you have in fighting this disease.
My name is Liz DeVivo, and I’m aware that I’m rare.